Exstrophy of the Bladder. I. Long-Term Results in a Series of 37 Cases Treated by Ureterosigmoidostomy

Vol. 114, July

THE JOURNAL OF UROLOGY

Printed in U.S.A.

Copyright © 1975 by The Williams & Wilkins Co.

EXSTROPHY OF THE BLADDER. I. LONG-TERM RESULTS IN A SERIES OF 37 CASES TREATED BY URETEROSIGMOIDOSTOMY HARRY M. SPENCE, WILLIAM W. HOFFMAN

AND

VIRGIL A. PATE

From the Department of Urology, The Dallas Medical and Surgical Clinic and the University of Texas Southwestern Medical School, Dallas, Texas

ABSTRACT

Of the 37 patients with exstrophy of the bladder in whom ureterosigmoidostomy was selected as the mainstay of treatment 4 died of causes related to the procedure. Faulty judgment and poor followup accounted for these deaths, which might well be preventable today. In 18 survivors, or approximately half of the entire series, the operation has held up well for many years, with no further surgical procedure being required for complications. In 12 patients, or approximately a third of the group, remedial operations for complications attributable to the ureterosigmoidostomy have been required but it was elected to retain this form of diversion. The long-term end result in these patients also remains acceptable. Combining these 2 groups 30 of 37 patients with ureterosigmoidostomy may be considered to have been eventually successful. However, in 7 of 37 patients the method was a frank failure, in that 4 patients died and 3 required substitution of another form of diversion. While all forms of treatment for exstrophy of the bladder leave much to be desired, results obtainable in today's setting lead us to recommend ureterosigmoidostomy as first choice in the management of this disorder. This recommendation is accompanied by the admonition that systematic followup is imperative so that if things do go badly from the clinical, laboratory or urographic viewpoint corrective measures can be done before renal deterioration occurs. The measures required may range from simple correction of electrolytes to conversion to an ilea! loop. For many years we have practiced simultaneous bilateral ureterosigmoidostomy as the preferred basic approach in the treatment of exstrophy except when obvious incompetence of the anal sphincter precluded use of the large bowel as a reservoir for the urine.* Clearly whenever any form of urinary diversion is done some disposition of the bladder must be made and the external genitalia must be reconstructed. Specialized techniques for cystectomy and for correction of the genitalia that we have found useful are described elsewhere. 1 • 2 We herein update and extend our previous report 3 by analyzing the long-term results achieved in 37 patients with exstrophy in whom it was chosen to divert the urine into the intact sigmoid by ureterocolic anastomosis. All living patients have been followed for a minimum of 5 years and most much longer. How have our patients fared early and late? Has the operation held up over the years? Answers to be found in the laboratory, urographic and clinical data on this group may provide helpful information for improving future results when this form of diversion is chosen as well

as affording a base line for comparison with series of patients treated by methods other than uretero-sigmoidostomy. MATERIAL STUDIED

One of us (H. M. S.) has been responsible for either all or a significant portion of the surgical care in each of the 37 patients (table 1). In 35 cases the ureterocolic anastomosis was done by the senior author personally, under his direct supervision or by close associates. One patient when seen originally had already suffered the surgical loss of a kidney but the remaining kidney and exstrophic bladder were untouched. Two patients had had bilateral ureterosigmoidostomy done elsewhere (in 1921 and 1934) but came under our care for major complications requiring operation and still remain under our supervision. Thus, we have available for review 73 renal units in 37 patients. Recent data are available on all patients so that the final outcome or current status is known at the time of this report (September 1, 1974). Previous operations for exstrophy. In 5 patients 1 Accepted for publication October 25, 197 4. or more attempts at anatomic closure had been * It is elementary that in all discussions involving carried out before the patient was seen by us. Two urinary diversion and bowel one must distinguish between· the bowel as conduit and the bowel as urinary were acceptable cosmetically but all were complete failures with respect to urinary control. Furtherreservoir. 133

134 TABLE

SPENCE, HOFFMAN AND PATE

1. Material studied: ureterosigmoidostomy basic

treatment in 37 patients No. Cases Sex:

M~ Female Age at operation: 3 mos. or less 4to9mos. 1 to4yrs. More than 4 yrs. Technique: Coffey I method Mucosa-to-mucosa plus tunnel or nipple antireflux component Bowel preparation after effective bowel sterilizing agents available Cystectomy and genital reconstruction Previously reconstructed bladder left in situ One or more prior attempts at functional closure

TI 10 14 9 9 5 5 32 35 35 2 5

more, in the 2 patients whose bladders had been turned in successfully significant bilateral reflux was persistent. One girl was made particularly miserable by erosion into the vagina of wire sutures used to approximate the pubes. Age factors. We had the opportunity of seeing many of these patients as newborns and the majority within the first few months of life. Aside from those instances in which ureterosigmoidostomy or anatomic closure had been performed elsewhere all patients were seen within the first 4 years of life except for a 13-year-old girl from a remote depressed rural area. The individuals who had undergone attempts at functional closure came to ureterosigmoidostomy 2, 3, 8 and 10 years after the original procedure had proved unsatisfactory. All patients seen early in life had completely normal upper tracts by urography and none had suffered overt urinary infection. Indeed, to our surprise only 1 instance of preoperative major upper tract damage was encountered in the entire group. As our early favorable experiences with ureterocolic anastomosis grew our standard approach evolved of recommending 1-stage bilateral ureterosigmoidostomy in any robust infant more than 3 months old with exstrophy if the tone of the anal sphincter seemed good on digital examination. Operative technique. Five cases were done by the Coffey I technique, making a submucosal tunnel but permitting the distal raw end of the ureter to dangle within the bowel lumen. In 32 cases a mucosa-to-mucosa anastomosis of the ureter and bowel was combined with some antireflux measure, either tunnel (Leadbetter) or nipple (modified Cordonnier). Interrupted fine catgut sutures are preferred for the inner mucosal layer with swedged on 5-zero arterial silk suture material for the serosa to adventitia approximation. Ureteral stents are regarded as unnecessary and undesirable. Only 2 cases were done before the .availability of effective bowel sterilizing drugs and antimicrobial agents.

RESULTS

Meticulous followup studies are essential in the evaluation of any form of management of exstrophy. In our series emphasis has been placed on the performance of excretory urograms (IVPs) and blood urea nitrogen (BUN), creatinine and electrolyte determinations every 3 to 6 months in the first postoperative year or until stability appears assured and annually thereafter. A cystokon enema is useful to make sure reflux is not present, as is a close look at the film of the kidneys, ureter and bladder for pneumopyelogram. In assessing the long-term results our 37 patients have been divided into 4 groups composed respectively of 1) deaths, 2) original ureterosigmoidostomy maintained unaltered, 3) subsequent surgical intervention required for complications but the initial type of diversion retained and 4) substitution of a different form of diversion necessary (table 2). Deaths. No hospital deaths occurred but 5 patients have died from 18 months to 19 years postoperatively, 4 of urologic causes and 1 of a traffic mishap (table 3). Death 1: J. C., a 6-month-old female infant, had undergone bilateral Coffey I transplant in 1945. About 9 years later recurrent urinary tract infections and pyelographic deterioration from obstruction at the anastomotic site developed. Anastomotic revision in the presence of chronically dilated ureters proved unavailing and the girl d'ed a few months later of an unrecognized acute electrolyte imbalance. Autopsy showed badly damaged kidneys. Death 2: K. C., a miserable 9-year-old girl with massively dilated upper tracts, had undergone several unsuccessful attempts at primary reconstruction and finally underwent bilateral cutaneTABLE 2. Duration of follow up and current status regarding original ureterosigmoidostomy in 33 surviving patients

No. Pts. Duration: 20 yrs. or more 10 to 19 yrs. 5 to 10 yrs. Bilat. ureterosigmoidostomy retained Unilateral ureterosigmoidostomy retained Another form of diversion necessary

TABLE

15 13 5 27 3

3

3. Deaths No. Cases

Attributable to ureterosigmoidostomy: 10 yrs. postop. 9 yrs. postop. 3 yrs. postop. 1 ½ yrs. postop. Poor choice of operation Inadequate followup Unrelated to ureterosigmoidostomy

4

2 2 1*

* Death from traffic accident 19 years after successful unile.teral ureterosigmoidostomy.

135

EXSTROPHY OF BLADDER

1

;

·) ,

'

ous ureterostomy in 1948. Management of the ureterostomies was a problem and we decided to perform an end-to-side bilateral ureterosigmoidoscopy in 1949. As should have been anticipated subsequent roentgenograms showed reflux of gas to the already severely damaged kidneys. A diversion colostomy was eventually performed but had been too long delayed and the patient died a year later when she was 18 years old of renal failure. Postmortem examination showed both kidneys to be mere shells filled with stones. Death 3: M. F., a 15-year-old boy with exstrophy, had suffered the surgical loss of a kidney elsewhere. In 1950 a ureterosigmoidostomy was chosen to divert the normal left kidney. He did well thereafter, undergoing cystectomy and genital reconstruction uneventfully in addition to the diversion. A pyelogram after 6 months was satisfactory but a significantly elevated BUN and decreased carbon dioxide were worrisome. The patient returned to his home in a remote rural area and was lost to followup. Eventually it was learned that he died suddenly of kidney infection 18 months postoperatively, probably in electrolyte crisis. Death 4: A. C., after several unsuccessful attempts to reconstruct the bladder in the newborn period, underwent ureterosigmoidostomy in 1956 when he was 2 years old. Convalescence was smooth but he was not seen again until 3 years later when his pediatrician admitted him to a hospital moribund from intractable vomiting and dehydration secondary to respiratory and urinary infections. Autopsy showed pyelonephritis, stones and hydronephrosis. Original ureterosigmoidostomy unaltered. The ureterosigmoidostomy was unaltered in 20 individuals, embracing 39 renal units. Two of these patients died of causes unquestionably related to the procedure. In the remaining 18 survivors the clinical state and quality of life remain good. Mild hyperchloremic acidosis is the rule but this is controlled readily by oral administration of Shohl's solution when the carbon dioxide combining power decreases to less than 20 mEq. Several patients have had minor infections responsive to ambulatory treatment but only 3 have required hospitalization for urinary tract infection. In none has infection been of significant magnitude to make us consider conversion to an ilea! loop. The urograms in the majority of this group maintain a consistently normal appearance from 5 to 25 years after the original procedure. Only 1 kidney causes concern now and in this patient since the urographic changes have remained stationary for several years nothing further is contemplated. Operation required for complications but ureterosigmoidostomy (unilateral or bilateral) has been retained (table 4). Of the 37 patients 13 or just more than a third have required subsequent operative procedures because of complications or failure of the original ureterosigmoidostqmy on either one or both sides. Nevertheless, in these patients it has

4. Operations for complications subsequent to ureterosigmoidostomy in 17 patients (22 procedures)*

TABLE

No. Cases Nephrectomy Ureterolithotomy Nephrolithotomy and resection lower pole Revision and/or reanastomosis Conversion to ilea] loop Cutaneous ureterostomy Diversion colostomy

4 5 2 7 2

* Calculi played a significant role either primary or secondary in 8 of the 17 patients requiring operation for complications.

been possible to retain this form of diversion after a corrective operation. In 5 patients revision of the initial anastomosis was done. Ureterolithotomy with or without such revision has been necessary in 4 instances, pyelolithotomy and segmental resection in 2 and nephrectomy in 3. What has been the outcome over the years in these patients in whom it was elected to continue with ureterosigmoidostomy after corrective operations for complications have been performed? One patient has died (death 1), 1 continues to have stone problems and 2 have significant permanent unilateral renal damage. Nevertheless, the 17 remaining renal units in 12 patients remain normal or nearly so on urography and all of the 12 individuals enjoy good health from 5 to 53 years after the original ureterocolic anastomosis. The interval between the original ureterosigmoi~ostomy and the time that operation for complications was actually performed ranged from 6 months to 10 years. Substitution of a different form of diversion necessary. Recurrent urinary tract infection associated with urographic deterioration in 3 patients prompted conversion to an ileal loop in 2 and cutaneous ureterostomy in 1 with a single kidney (table 4). A diversion colostomy was done in another patient but had been delayed far too long and was ineffective (death 2). Clinical responses have been gratifying in the 3 patients who were formally converted from ureterosigmoidostomy and the urographic results show that progressive damage has been halted. The findings on IVP after ureterosigmoidostomy are tabulated in table 5. The examinations are done without preparation other than nothing by mouth for 6 hours prior to the test. The incidence of infection after ureterosigmoidostomy. Major infection occurred at some time after the original operation in almost half (17 of 37) of the entire series (table 6). Minor infections were less frequent (9 of 37), yet it is noteworthy that in a third no demonstrable infection was encountered. Electrolyte and blood chemical disturbances. Evaluation of the laboratory data in our long-term survivors confirms our previous findings that a tendency toward slight lowering of the carbon dioxide combining power along with an elevation of the blood chloride level may be anticipated al-

136

SPENCE, HOFFMAN AND PATE TABLE

17 1 13 5

Acceptable control has been present in all but 1 gcrl who requires protective padding. Frequent diaper change in infancy with emphasis on good perinea! hygiene thereafter is obviously necessary. Finally the psychological status of patient and parents has impressed us as being superior to that noted with other forms of diversion. This status is particularly true if the ureterosigmoidostomy is performed early in life.

No. Units

DISCUSSIO:'i

5 No. Pts.

Postoperative urography after initial ureterosigmoidostomy in 36 patients prior to operations for complications: Both sidea good Solitary kidney good One side good/opposite side poor Both sides poor

Urographir findings by individual: No. kidneys Good urographically Poor

71 48

23 No. Units

X-ray findings after secondary operation for complications but with ureterosigmoidostomy retained: No. kidneys G~* Poort

12 7 5

• No or minimal dilation of collecting system, prompt excretion in adequate concentration of urographic medium and no stones. t Abnormal collecting system, calculi and inadequate function as judged by appearance, time and concentration of radiographic medium. TABLE

Three methods of treatment are in general use for the management of exstrophy of the bladder: 1) anatomic closure, 2) ilea! loop diversion and 3) ureterosigmoidostomy. The first procedure retains a potentially dangerous epithelium, is prone to upper tract deterioration and yields disappointing results in the percentage of patients who achieve true urinary control.'· 5 The ilea! conduit which has become justifiably popular as an effective form of permanent upper tract diversion is by no means free of complications, major or minor, and carries the obvious drawback of requiring an external device. However, when the loop was introduced a number of our patients were doing so well with ureterosigmoidostomy that we elected to continue its use. In retrospect this choice seems warranted in our

6. Infection problem

37 patients after original ureterosigmoidostomy 17 patients after corrective operations for complications

Major*

Minert

Nonet

17

9

11

8

7. Blood chemical findings 6 years or more postoperatively in 29 living patients with ureterosigmoidostomy

TABLE

No. Cases

9

* Average of 2 or more attacks per year and acutely ill often requiring hospitalization. t Less than 2 attacks per year, mildly ill and no hospitalization. t No history of loin pain, fever, malaise or unexplained symptoms.

though this is by no means invariable (table 7). From the clinical viewpoint these findings are surprisingly asymptomatic. However, if the carbon dioxide is less than 20 mEq. or the chloride level is more than 110 mEq., correction by the judicious use of an oral alkalizing agent is indicated. Likewise a modest elevation of the BUN is a frequent finding but usually the accompanying normal serum creatinine level is reassuring in regard to renal function. Potassium and sodium levels are rarely abnormal as are the calcium and phosphorus concentrations. Other parameters in the over-all clinical appraisal. Other parameters include growth and development, rectal control and the psychological status (table 8). We have no firm figures on the growth rate, although our impression is that the patients with ureterosigmoidostomy may be somewhat smaller than their contemporaries. We have relied preoperatively on simple digital examination of the anal sphincter to predict its competence.

BUN determinations in 29 patients (normal range 10 to 20 mg.%): Wm~%~d~u 21 to 32 mg.% Creatinine values in 23 patients (normal range 0.5 to 1.2 mg. %): 1.2 mg.% or less 1.5 and 1.9 mg.% Carbon dioxide combining power in 29 patients (normal range 24 to 32 mEq. per 1.): 24 mEq. per 1. or more 19 to 23 mEq. per 1. Less than 19 mEq. per 1. Blood chloride determinations in 28 patients (normal range 99 to 108 mEq. per 1.): 100 to 108 mEq. per 1. 109 to 114 mEq. per 1. 117mEq.perl.

TABLE

IB 14

21 2

17 10 2

18 9 1

8. Over-all appraisal of 37 patients treated with ureterosigmoidostomy* No. Caser;

Acceptable resultt: Original anastomosis intact, 17 Required secondary operation, 13 Failure (including 4 deaths)t

30

7

• Clinical status, IVPs and laboratory data. regret on part of patient or doctor; doubtful if patient would have fared better with another approach. t Ureterosigmoidostomy abandoned or death related to procedure.

t No

137

EXSTROPHY OF BLADDER

series and is supported by the long-term evaluations of others which are now appearing in the literature. 6 • 7 From a review of these it is apparent that a combination of factors accounts for the improved outlook in a patient with ureterocolic anastomosis that is obtainable today as compared to former times. First, techniques combining mu' cosa-to-mucosa approximation between ureter and · bowel with an antireflux component added are clearly superior. Conversely, implantation of dilated ureters into intact colon either initially or as a salvage effort is questionable and the hazard increases with the degree of ureterectasis. Under such circumstances the ileal loop is our choice now. However, frequent IVPs may detect beginning dilation early enough to permit successful anastomotic revision. Within the last 2 decades along with improved techniques the development of effective antimicrobials and an understanding of electrolyte physiology have changed the picture immeasurably for the better after ureterosigmoidostomy. Although half of our patients have at no time exhibited evidence of significant clinical or urographic pyelonephritis it definitely remains a potential threat. It is here that aggressive antimicrobial and rational parenteral fluid therapy of infection in the acute phase followed by long-term suppressive medication has lessened dramatically the occurrence of renal damage, especially when the kidneys are urographically normal. We have found it illadvised to rely on such conservative measures when obstruction or stones complicate the picture. Here appropriate early surgical intervention is indicated. A mild degree of hyperchloremic acidosis is encountered frequently and appears innocuous. Nonetheless, the clinician must be alert to sudden and marked alterations associated with renal, respiratory or gastrointestinal infectious episodes. Severe acidosis and hypokalemia will be lethal unless effective electrolyte reconstitution is undertaken promptly. These facts must be impressed upon primary physicians and patients (or

parents) who are often unaware of the unique and insidious hazards which ureterosigmoidostomy presents. Ranking in importance with all of the foregoing in achieving successful long-term results after ureterosigmoidostomy is a systematic followup program. Inherent hazards peculiar to this form of diversion make monitoring of electrolytes and close surveillance of urograms imperative. The IVP unquestionably has furnished the most valuable information in our postoperative followup protocol. In general prompt excretion of the contrast medium by unobstructed kidneys presages a good over-all result while hydronephrosis, ureterectasis, calculi and poor drainage go hand in hand with clinical infection and metabolic disturbances. An important point is whether on serial examinations minimal changes are stationary or progressively worsening. Systematic reappraisals in the light of our current knowledge will disclose insidious renal deterioration sufficiently early to permit medical and surgical corrective measures. REFERENCES

1. Spence, H. M.: A simplified technique for cystectomy

2. 3. 4. 5. 6. 7.

and repair of the abdominal defect in exstrophy of the bladder. J. Urol., 77: 428, 1957. Hoffman, W. W. and Spence, H. M.: Management of exstrophy of the bladder. South. Med. J., 58: 436, 1965. Spence, H. M.: Ureterosigmoidostomy for exstrophy of the bladder. Results in a personal series of thirtyone cases. Brit. J. Urol., 38: 36, 1966. Marshall, V. F. and Muecke, E. C.: Functional closure of typical exstrophy of the bladder. J. Urol., 104: 205, 1970. Megalli, M. and Lattimer, J. K.: Review of the management of 140 cases of exstrophy of the bladder. J. Urol., 109: 246, 1973. Wear, J. B. and Barquin, 0. P.: Ureterosigmoidostomy: long-term results. Urology, 1: 192, 1973. Bennett, A. H.: Exstrophy of bladder treated by ureterosigmoidostomies: long-term evaluation. Urology, 2: 165, 1973.